黄葵胶囊联合缬沙坦治疗IgA肾病疗效观察

IgA肾病是临床上常见的一种肾脏疾病,是亚洲人群中引起血尿原因最常见的肾小球疾病。尿蛋白是临床上判断疾病严重程度及进展的有效指标。24h尿蛋白定量〉3g的IgA肾病患者,应用激素治疗具有较多的循证医学证据;对于24h尿蛋白定量在1～3g的患者,是否应用激素治疗存在较大争议;24h尿蛋白定量〈1g者,多数学者认为不用激素类药物。本观察应用黄葵胶囊联合缬沙坦治疗IgA肾病24h尿蛋白定量〈1g患者,观察对尿蛋白及血压的影响。     

IgA肾病牛津分型在判断肾脏预后中的作用

目的:利用IgA肾病患者的临床随访资料,评价牛津分型在判断IgA肾病预后中的作用.方法:随访的患者均经过肾活检确诊为IgA肾病.收集患者的一般状况及实验室检查结果,简化MDRD公式计算估计肾小球滤过率(eGRF).肾脏病理按照IgA肾病牛津分型(MSET)评分.统计均采用SPSS软件计算.结果:共纳入122例原发性IgA肾病患者,随访时间3~196月,女性患者占56.6%.系膜增生(M)、肾小管萎缩/间质纤维化(T)和肾穿时的临床指标存在显著相关.仅T和终点事件存在相关性.肾穿时激素使用组的肾功能、尿蛋白定量差于非激素使用组,但随访结束,两组间的肾功能、尿蛋白定量无明显差异.结论:T对评价肾脏预后具有显著价值,激素治疗可能具有延缓IgA肾病进展的作用.     

针灸治疗IgA肾病的作用

目的观察针灸治疗对IgA肾病患者蛋白尿、血肌酐（SCr）、血浆内皮素（ET）的影响，探讨针灸对IgA肾病的治疗作用。方法IgA肾病患者30例，随机分为两组，针灸联合激素组15例，接受针灸联合口服泼尼松龙治疗；针灸组15例仅接受针灸治疗，疗程均为2个月。测定治疗前、后24h尿蛋白定量、SCr、血浆ET浓度。结果治疗后两组患者24h尿蛋白定量、SCr、血浆ET浓度均较治疗前显著降低，针灸联合激素组降低的幅度显著高于单纯针灸治疗组（P〈0．01）。结论针灸治疗IgA肾病有减少蛋白尿、保护肾功能的作用，此可能与降低血浆ET水平有一定关系，针灸联合激素治疗IgA肾病的效果优于单纯使用针灸。     

邱模炎教授从“调补分化”论治IgA肾病经验

<正>IgA肾病是我国最常见的原发性肾小球肾炎,研究发现部分IgA肾病呈进行性进展,确诊10~20年后,约有15%~40%的患者发展为终末期肾脏病(ESRD)[1]。IgA肾病最常见的临床表现为发作性肉眼血尿和无症状性血尿和(或)蛋白尿[2]。目前西医对于IgA肾病多采用免疫抑制剂、激素以及对症治疗,临床疗效欠佳,预后亦非乐观。因此,对于IgA肾     

IgA肾病的激素治疗:对大量蛋白尿病例的回顾性研究

IgA肾病(IgA—N)是常见的原发性肾小球肾病,30—40％的患者经过缓慢的病程进展后发生终未期肾衰。作者对经激素治疗的IgA—N的IgA—N的临床病程进行了随访观察,评价了激素治疗的作用。病人与方法 29例伴有大量蛋白尿(尿蛋白≥2.0/日)和34例中11例伴有中等量蛋白尿(尿蛋白1.0—2.0/日)的IgA—N患者接受了1—3羊激素治疗,最初用强的松40mg/日,持续4周,以后递减至30、25、20mg/日各8周,维持量为15     

IgA肾病合并膜性肾病一例

报告1例IgA肾病合并膜性肾病.患者临床肾损害表现为蛋白尿、血尿及肾功能异常,肾组织病理诊断为IgA肾病合并抗磷脂酶A2受体抗体(PLA2R)相关性膜性肾病.半乳糖缺乏IgA1(Gd-IgA1)特异性抗体阳性,亦排除了IgA非特异性沉积的可能.经激素及血管紧张素受体拮抗剂(ARB)等药物治疗后病情快速缓解.提示对于膜性肾病合并肾组织有IgA沉积的患者,除了需排除继发性膜性肾病,鉴别IgA是否为特异性沉积亦具有重要意义,避免不必要的免疫抑制剂使用.     

糖皮质激素治疗中等量蛋白尿IgA肾病的系统评价

IgA肾病目前尚缺乏统一有效的治疗方案.激素治疗中等量蛋白尿IgA肾病一直是关注和争论的焦点.我们对近年有关的文献进行系统评价,以探讨其长期疗效及安全性,为IgA肾病的治疗寻找合理、长期有效的方案提供依据.     

检测IgA-CIC对IgA肾病进行鉴别性分析的意义

IgA肾病的特点是在肾小球系膜区有IgA沉积,并有少量IgA、IgG-IgM和C3沉积。IgA肾病须经肾活检来确诊,但由于种种原因肾活检尚难以普及。本文旨在探讨不进行肾活检的情况下,怎样通过临床指标来鉴别IgA肾病,并寻求对鉴别IgA肾病与非IgA肾病的最有意义的临床指标。病人和方法:观察了56例IgA肾病患者和54例非IgA肾病的原发性慢性肾小球肾炎患者。IgA肾病组,男40例,女16例,年龄15.9～55.5岁;非IgA肾病组,男36例,女18例,平均14.1～68.6岁。所有患者均经肾活检明确诊断。用于IgA肾病与非IgA肾病鉴别分析的21项临     

来氟米特联合激素治疗伴肾小管间质损害的IgA肾病临床观察

目的：研究来氟米特联合糖皮质激素治疗伴肾小管间质损害的IgA肾病的疗效和安全性。方法：选取原发性IgA肾病伴轻到中度肾小管间质损伤的患者25例。采用Katafuchi的半定量标准对其进行组织学评分,用来氟米特联合中等剂量激素治疗至少6个月,分别观察患者治疗3月、6月和1年时的24h尿蛋白定量、肾功能、血清白蛋白和药物副作用。结果：随访期末完全缓解21例（84.0%）,部分缓解2例（8.0%）,无效2例（8.0%）;治疗6个月时上述指标均有明显改善。肾小管间质病变积分较高的患者疗程相对较长。结论：来氟米特联合激素治疗伴肾小管间质损伤的IgA肾病患者具有较好的疗效且副作用较小。     

肾脏病理高积分的少量蛋白尿IgA肾病激素治疗疗效观察

目的：观察肾脏病理高积分的少量蛋白尿IgA肾病患者给予激素治疗的疗效。方法：45例少量蛋白尿IgA肾病患者随机分为两组,所有患者病例积分Haas分型≥Ⅱ型、Katafuehi积分肾小球损伤积分≥2分和（或）肾小管间质损伤积分≥2分,治疗组（22例）给予激素和常规治疗,对照组（23例）仅给予常规治疗。结果：治疗组尿蛋白量显著减少[（0．48±0．17）g／24hvs（0．93±0．36）g／24h,P〈0．05];中等量蛋白尿、eGFR下降终点事件发生率显著低（P〈0．05）。结论：以肾脏病理积分作为少量蛋白尿IgA肾病的指导依据可以获得更好临床预后。     

Discriminant analysis of clinical markers before renal biopsy in patients with IgA nephropathy

Discriminant analysis of clinical markers before renal biopsy in patients with IgA nephropathy is described. Sixty eight patients with IgA nephropathy (IgA nephropathy group) and 66 patients with other chronic glomerulonephritis (non-IgA nephropathy group) were examined. The discriminant analysis was applied to separate those two groups by using twenty clinical parameters as well as binding capacity of serum IgA to the glomeruli of renal specimens. Binding of serum IgA of patients to the glomeruli obtained from patients with IgA nephropathy was performed using avidin-biotin immunofluorescence. Among twenty clinical markers, the levels of serum IgA and creatinine, and degree of microhematuria in IgA nephropathy group were significantly higher than those in non-IgA nephropathy group Furthermore, the positive incidence of serum IgA binding of IgA nephropathy group was significantly higher than that of serum IgA binding of non-IgA nephropathy group. The correct classification rate were 79.10% using five clinical markers including serum IgA, microhematuria, serum C4, quantitation of proteinuria and degree of proteinuria. It is indicated that the levels of serum IgA and the binding of serum IgA to the glomeruli were considered to be major markers for clinical diagnosis of patients with IgA nephropathy It was concluded that the discriminant analysis before renal biopsy was useful for diagnosis of IgA nephropathy.     

Serum levels of galactose-deficient IgA in children with IgA nephropathy and Henoch-Schönlein purpura

IgA nephropathy and Henoch-Schönlein purpura nephritis (HSPN) are related diseases characterized by deposits of IgA1-containing immune complexes in the renal mesangium. Adult patients with IgA nephropathy have aberrantly glycosylated IgA1 (galactose-deficient O-linked glycans) in the circulation and renal deposits. However, IgA1 glycosylation has not been studied in pediatric patients with IgA nephropathy. Using our quantitative lectin enzyme-linked immunosorbent assay (ELISA) test, we measured serum levels of galactose-deficient IgA1 of children with IgA nephropathy and HSPN and controls. Children with IgA nephropathy and HSPN had serum levels higher than those of healthy children or renal-disease controls with C1q nephropathy. Furthermore, lectin ELISA identified patients with HSPN whose clinical course mimicked that of IgA nephropathy. In summary, pediatric patients with IgA nephropathy and HSPN have an aberrancy in the glycosylation in IgA1 O-linked glycans that is similar to that in adults with IgA nephropathy.     

补体在IgA肾病发生发展中的作用

IgA肾病的发生与自身免疫相关，其特征为肾小球系膜区IgA沉积。IgA肾病的发病机制尚不清楚，但现已证实补体异常活化在IgA肾病发生发展中起重要作用，参与IgA肾病发生发展过程的主要为补体替代途径和凝集素途径。本文就补体系统在IgA肾病发生发展中的作用进行了综述。     

Effect of immunoglobulin depositions of glomerular sialic acids in patients with IgA nephropathy

A study of double immunofluorescence-staining of immunoglobulins and sialic acids in the glomeruli from patients with IgA nephropathy is described. Renal biopsy specimens from patients with IgA nephropathy were stained with rhodamine-labeled antihuman IgA, IgG or IgM antisera and then stained with FITC-labeled Limulus polyphemus (LPA), Tricum vulgaris (WGA) or antihuman C3 antisera. Marked positive stainings of IgA and C3 and positive binding of LPA or WGA were observed in the glomerular mesangial areas from patients with IgA nephropathy. LPA or WGA were not bound with glomerular capillary walls from patients with moderate and advanced stages of IgA nephropathy, although depositions of IgA and C3 were markedly observed in such walls. There was a significant inverse correlation between the deposition of IgA and the binding of LPA or WGA in glomerular capillary walls obtained from these patients with IgA nephropathy. The levels of proteinuria from patients with moderate and advanced stages of IgA nephropathy were significantly higher than those with minimal and slight stages of such disease. It is suggested that the decrease of sialic acids in glomerular capillary walls might be due to a deposition of IgA in some patients with IgA nephropathy. It is concluded that high levels of proteinuria might be due to the decrease of sialic acids in glomerular capillary walls from patients with moderate and advanced stages of IgA nephropathy.     

Familial IgA nephropathy.

To date, more than 90 families with multiple members with IgA nephropathy have been reported. The case for genetic predisposition as a cause of familial clustering of IgA nephropathy is supported by several factors, including variable time points in the onset of the disease in relatives with IgA nephropathy; the presence of abnormalities of IgA production in both affected and unaffected family members; the clustering of the birthplaces of ancestors of large pedigrees containing multiple affected members with IgA nephropathy, which suggests the existence of a "founder effect". Immunogenetic studies does not conclusively indicate that HLA is involved in the pathogenesis of IgA nephropathy. The specific mode of inheritance of familial IgA nephropathy is difficult to establish with certainty. IgA nephropathy may be a single gene trait with incomplete penetrance. Alternatively, a multifactorial genetic disease could account for the increased prevalence of the disorder among relatives of affected individuals. Clinicians must become aware that IgA nephropathy may aggregate within families in a substantial number of cases. The availability of multiplex families offers an ideal opportunity to design a molecular genetics approach to map the gene(s) or pathway(s) responsible for the development of IgA nephropathy.     

Significance of levels of circulating IgA-class immune complex in discriminant analysis of patients with IgA nephropathy before renal biopsy

Discriminant analysis of clinical markers including circulating IgA-class immune complex (IgA-CIC) before renal biopsy in patients with IgA nephropathy is described. Fifty-six patients with IgA nephropathy (IgA nephropathy group) and 54 patients with other primary chronic glomerulonephritis (non-IgA nephropathy group) were examined. Discriminant analysis was applied to separate these two groups by using 21 clinical markers including levels of IgA-CIC. The levels of IgA-CIC in sera were measured by a solid-phase anti-C3 Facb enzyme immunoassay (EIA). Among these clinical markers, the levels of serum IgA, IgA-CIC and creatinine, and the degree of microhematuria in the IgA nephropathy group were significantly higher than those in the non-IgA nephropathy group. Contributions of IgA and IgA-CIC to the classification were very high and both had almost the same effect. The correct classification rate was 80.00% using five clinical markers: serum IgA, microhematuria, IgA-CIC, serum creatinine, and blood urea nitrogen. It was shown that the levels of serum IgA and IgA-CIC were major markers for the clinical diagnosis of patients with IgA nephropathy. It was concluded that discriminant analysis before renal biopsy was useful for the diagnosis of IgA nephropathy.     

Serum IgA/C3 ratio may predict diagnosis and prognostic grading in patients with IgA nephropathy

Recently, the authors reported that the ratio of serum IgA to C3 (serum IgA/C3 ratio) is a good marker to distinguish patients with IgA nephropathy from non-IgA nephropathy patients together with serum IgA levels using an international reference preparation (IFCC/CRM470). In this study, the authors investigated whether the serum IgA/C3 ratio might be an indicator of prognostic grading in patients with IgA nephropathy. Two hundred and thirteen patients with IgA nephropathy and 96 other glomerular diseases including diffuse or focal mesangial proliferative glomerulonephritis without mesangial IgA deposition (non-IgA PGN), membranous nephropathy and thin basement membrane syndrome were examined. The levels of serum IgA and C3 in these patients were adjusted by the specified formula to those using international standard serum (IFCC/CRM470) in this study. The results of this study showed the highest levels of IgA/C3 ratio in patients with IgA nephropathy. The serum IgA/C3 ratio appears to gradually increase according to the prognostic grading of this disease. Therefore, measurement of the serum IgA/C3 ratio may be useful for prediction of diagnosis and prognostic grading in patients with IgA nephropathy.     

Measurement of secretory IgA in salivary juice and the localization of secretory IgA in duodenal mucosa in patients with IgA nephropathy

In order to clarify whether or not the local IgA immune system plays a role on the pathogenesis of IgA nephropathy, we measured serum levels of IgA, serum and salivary levels of secretory IgA in patients with IgA nephropathy and in healthy subjects, and compared them with the results of immunohistochemical study of the duodenal mucosa obtained from patients with IgA nephropathy and healthy subjects. The results were as follows. 1. The serum levels of IgA were significantly higher in patients with IgA nephropathy than in healthy subjects. 2. The salivary levels of secretory IgA were significantly higher in patients with IgA nephropathy and non IgA nephropathy than in healthy subjects. 3. There was no significant difference in the serum levels of secretory IgA between patients with IgA nephropathy and healthy subjects, but they were significantly higher in patients with hematuria than without hematuria. 4. The salivary levels of secretory IgA were lower in some patients with IgA nephropathy, in spite of the intense staining of the secretory component and J chain in the duodenal mucosa, than in healthy subjects. On the other hand, the salivary levels of secretory IgA were lower in other patients with IgA nephropathy, in addition to the weak staining of secretory component and J chain in the duodenal mucosa than in healthy subjects. These results suggested that the disorder of IgA mucosal immunity may contribute to the pathogenesis of IgA nephropathy.     

Th1 polarization in murine IgA nephropathy directed by bone marrow-derived cells

IgA nephropathy is the most common form of progressive glomerulonephritis although the pathophysiology of this nephropathy is unclear. The ddY mouse is a spontaneous animal model with variable incidence and extent of glomerular injury mimicking human IgA nephropathy. Here, we transplanted bone marrow cells from 20-week-old ddY mice with beginning or quiescent IgA nephropathy into irradiated similar ddY mice, C57Bl/6 (Th1 prone) mice, or BALB/c (Th2 prone) mice. Serum IgA/IgG complex and Th1/Th2 polarization of spleen cells was determined by enzyme-linked immunosorbent assay and confirmed by fluorescent cytometric analysis. The ddY mice with commencing IgA nephropathy demonstrated strong polarization toward Th1, while those with quiescent disease were Th2 polarized. Serum levels of IgA/IgG2a immune complex significantly correlated with the severity of the glomerular lesions. Bone marrow taken from mice with commencing IgA nephropathy conferred IgA nephropathy with Th1 polarization in recipient-quiescent mice, while transplantation from the quiescent mice ablated glomerular injury and mesangial IgA/IgG deposition in those commencing IgA disease. However, adoptive transfer of CD4(+) T cells from those whose disease began failed to induce any IgA deposition or renal injury. Our study suggests that bone marrow cells, presuming IgA producing cells, may initiate this disease. Th1 cells may be involved in the pathophysiology of the disease after glomerular IgA deposition.     

Oral challenge with cow's milk in patients with IgA nephropathy--estimation of serum antibodies to cow's milk protein]

The author investigated the serum levels of antibodies against casein, beta-lactoglobulin and lactalbumin before and after challenging with cow's milk in 35 patients with IgA nephropathy, 18 with primary glomerulonephritis except for IgA nephropathy (GN control) and 11 healthy volunteers (H control). Blood samples were obtained at fasting, and at 30, 60, 120 and 180 min after oral challenging with 400 ml of cow's milk. IgA and IgG anti-cow's milk proteins antibodies were analyzed by ELISA. The same challenge was tested after administration of the antiallergic agent, sodium cromoglycate (SCG), in 11 patients with IgA nephropathy and 4 H controls. Serum levels of IgA anti-casein, -beta-lactoglobulin and lactalbumin antibodies in patients with IgA nephropathy were significantly higher than in control groups before challenging. However, those of IgG antibodies were not. The percent change of antibody titer after challenging with cow's milk did not elevate in any group, except for the level of IgA anti-beta-lactoglobulin antibody at 60 min in IgA nephropathy. Cases in which challenging produced marked elevation above the M + 2SD of the levels found in H control were expressed as "positive". The number of "positive" cases was 16 (45.7%) with IgA nephropathy, but none with GN control. There was no significant correlations between "positive" and "negative" cases with IgA nephropathy in clinical manifestations. In 3 out of 4 "positive" patients with IgA nephropathy, the levels of IgA antibody were suppressed after administration of SCG. It is concluded that the serum levels of IgA antibodies against cow's milk proteins are significantly elevated in IgA nephropathy, and are inhibited in elevation after oral challenge with cow's milk by administration of an antiallergic agent in some patients with IgA nephropathy.